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Early Versus Staged Repair Of Pulmonary Atresia With Ventricular Septal Defect And No MAPCAs; A Pediatric Health Information System Analysis
Mohamed Aashiq Abdul Ghayum, MBBS, James E. O'Brien, MD, Edo K. S. Bedzra, MD.
Children's Mercy Kansas City, Kansas City, MO, USA.

ObjectiveThe practice patterns and management strategies of patients with Pulmonary Atresia and Ventricular Septal Defect (PA/VSD) without Major Aortopulmonary Collaterals (MAPCAs) are not well reported. We sought to describe choice and outcomes of different interventions. MethodsPatients with diagnosis of PA/VSD without MAPCAs with two ventricle strategy between 2003-2023 were identified. Patients who underwent early single stage correction were compared to those staged to repair with a ductal stent, modified BTT shunt (BTTS) or RV-PA connection using Kruskal-Wallis test and Fisher’s exact tests.ResultsThe first intervention was single-stage repair in 135, Ductal stent in 41, BTTS in 97, and RV-PA connection in 31 patients. In-hospital mortality differed between the groups (11.85% vs 4.88% vs 2.06% vs 9.68%, p=0.02) as did ECMO rates (Single-stage 8.89%, Stent 2.44%, BTTS 3.09%, RV-PA 19.35%, p=.015). 22, 82, and 21 patients in the Stent, BTTS, and RV-PA groups achieved full repair with In-hospital mortality of 4.55%, 0%, and 4.76%; and ECMO rates of 4.55%, 7.32%, and 23.81% respectively. There was an overall positive trend in the utilization of early single-stage repair over the study period; nearly 60% of interventions in 2023 (Figure 1). ConclusionSingle-stage and staged repair of patients with PA/VSD without MAPCAs can be performed with acceptable outcomes. RV-PA connection may not be the favored palliative strategy for staged repair.
Figure 1: Practice Pattern for Initial Intervention over last 20 years

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